Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with sy...Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping,syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa ,which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.展开更多
例1、2 同卵双胎姐妹,4日龄,因“生后皮肤瘤样改变4d”于2016年9月收入复旦大学附属儿科医院新生儿科,分别系第1胎第1产和第1胎第2产,出生胎龄36“周,出生体重分别为2.60、2.45 kg,自然生产,有胎膜早破病史,1、5、10 min Apgar评分为7、...例1、2 同卵双胎姐妹,4日龄,因“生后皮肤瘤样改变4d”于2016年9月收入复旦大学附属儿科医院新生儿科,分别系第1胎第1产和第1胎第2产,出生胎龄36“周,出生体重分别为2.60、2.45 kg,自然生产,有胎膜早破病史,1、5、10 min Apgar评分为7、8、10分（两例一致）.出生后两例患儿全身即出现大小不一红斑,质韧,凸出皮面,局部皮肤可见大疱、结痂,局部大疱伴破溃渗出,颜面部可见密集肉瘤样赘生物.两患儿临床表现基本相同,生后曾于当地医院予“皮肤护理、抗感染”等治疗（具体不详）,皮肤病变无好转,遂于生后第4天由当地医院转入我院新生儿重症监护病房进一步多学科诊治.其母幼时曾有吃海鲜后皮肤出现红色丘疹过敏史,孕期进食鸡蛋、海鲜较多,孕期母亲未出现皮疹、红斑等过敏症状.其父及祖父母均无过敏史.展开更多
文摘Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping,syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa ,which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.
文摘例1、2 同卵双胎姐妹,4日龄,因“生后皮肤瘤样改变4d”于2016年9月收入复旦大学附属儿科医院新生儿科,分别系第1胎第1产和第1胎第2产,出生胎龄36“周,出生体重分别为2.60、2.45 kg,自然生产,有胎膜早破病史,1、5、10 min Apgar评分为7、8、10分（两例一致）.出生后两例患儿全身即出现大小不一红斑,质韧,凸出皮面,局部皮肤可见大疱、结痂,局部大疱伴破溃渗出,颜面部可见密集肉瘤样赘生物.两患儿临床表现基本相同,生后曾于当地医院予“皮肤护理、抗感染”等治疗（具体不详）,皮肤病变无好转,遂于生后第4天由当地医院转入我院新生儿重症监护病房进一步多学科诊治.其母幼时曾有吃海鲜后皮肤出现红色丘疹过敏史,孕期进食鸡蛋、海鲜较多,孕期母亲未出现皮疹、红斑等过敏症状.其父及祖父母均无过敏史.
